Page 171 - Haematologica Atlas of Hematologic Cytology

CHAPTER 17 - - Mature B-cell neoplasms
Figure 26 Mantle cell lymphoma (MCL) (A and B) Peripheral blood smear from an an an elderly patient with conspi- cuous splenomegaly and leukocytosis Circulating lymphoid cells cells are very heterogeneous Some cells cells look like small lymphocytes lymphocytes Others are more similar to prolymphocytes A portion of the the cells have irregular nuclear borders and and are similar to centrocytes These cells expressed CD19 CD20 CD5 and and FMC7 but lacked CD10 CD23 and TdT Monoclonal surface immunoglobulin with with chain was expressed with with moderate intensity Cytogene- tic studies showed t(11 14)(q13 q32) while Cyclin D1 immunostain revealed nuclear positivity BCL-2 and c-MYC rearrangements were absent The distinctive cytologic immunophenotypic and cytogenetic features suggested the the diagnosis of of leukemic MCL MCL this was confirmed by histological analysis of of the the spleen MCL MCL is is is is is a a a a a a a rare subtype of of of lymphoma which originates from a a a a a a peripheral B cell of of of the inner mantle zone It is composed of of of small- to me- dium-sized lymphoid cells with slightly to markedly irregular nuclei morphologically resembling centrocytes or or sometimes lymphocytes MCL is associated with the the t(11 14)(q13 q32) involving the the immunoglobulin heavy chain and the Cyclin Cyclin D1 genes (BCL-1) resulting in in in in deregulated expression of Cyclin Cyclin 1D Figure 27 Mantle cell lymphoma blastoid variant A A A 69-year-old woman presented with weight loss remarkable
AB hepatomegaly and and splenomegaly A blood count showed anemia severe thrombocytopenia and and leukocytosis (WBC 33 4x109/L] (A) Peripheral blood smear showing pleomorphic cells ranging from small lymphocytes with irregular irregular nuclei to to to medium-sized cells with high nuclear:cytoplasmic ratio irregular irregular nuclear nuclear contours fine chro- matin and often prominent nucleoli (B) Bone marrow aspirate shows massive infiltration by numerous blast-like cells Immunophenotypic and cytogenetic findings were consistent with the diagnosis of MCL The blastoid va- riant of of MCL is is is characterized by cells with blast-like features and poor prognosis The differential diagnosis of of its leukemic leukemic phase includes acute leukemias prolymphocytic leukemia leukemia and other types of lymphomas in in leukemic leukemic phase 158